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BCKDHB Antibody (N-term) Blocking peptide
Synthetic peptide
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
| Primary Accession | P21953 |
|---|---|
| Clone Names | 90121079 |
| Gene ID | 594 |
|---|---|
| Other Names | 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial, Branched-chain alpha-keto acid dehydrogenase E1 component beta chain, BCKDE1B, BCKDH E1-beta, BCKDHB |
| Format | Peptides are lyophilized in a solid powder format. Peptides can be reconstituted in solution using the appropriate buffer as needed. |
| Storage | Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C. |
| Precautions | This product is for research use only. Not for use in diagnostic or therapeutic procedures. |
| Name | BCKDHB (HGNC:987) |
|---|---|
| Function | Together with BCKDHA forms the heterotetrameric E1 subunit of the mitochondrial branched-chain alpha-ketoacid dehydrogenase (BCKD) complex. The BCKD complex catalyzes the multi-step oxidative decarboxylation of alpha-ketoacids derived from the branched-chain amino-acids valine, leucine and isoleucine producing CO2 and acyl-CoA which is subsequently utilized to produce energy. The E1 subunit catalyzes the first step with the decarboxylation of the alpha-ketoacid forming an enzyme-product intermediate. A reductive acylation mediated by the lipoylamide cofactor of E2 extracts the acyl group from the E1 active site for the next step of the reaction. |
| Cellular Location | Mitochondrion matrix |
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Provided below are standard protocols that you may find useful for product applications.
Background
Branched-chain keto acid dehydrogenase is a multienzymecomplex associated with the inner membrane of mitochondria, andfunctions in the catabolism of branched-chain amino acids. Thecomplex consists of multiple copies of 3 components: branched-chainalpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2)and lipoamide dehydrogenase (E3). This gene encodes the E1 betasubunit, and mutations therein have been associated with maplesyrup urine disease (MSUD), type 1B, a disease characterized by amaple syrup odor to the urine in addition to mental and physicalretardation, and feeding problems.
References
Rose, J.E., et al. Mol. Med. 16 (7-8), 247-253 (2010) :Gorzelany, K., et al. Turk. J. Pediatr. 51(2):97-102(2009)Quental, S., et al. Mol. Genet. Metab. 94(2):148-156(2008)Kang, H., et al. Fertil. Steril. 89(3):728-731(2008)Flaschker, N., et al. J. Inherit. Metab. Dis. 30(6):903-909(2007)
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